Background MCTD is a rare connective tissue disease with many controversies regarding its recognition, clinical course and assessment. The clinical expression of MCTD is the consequence of its complex immunopathology. The chronic systemic inflammation leads to blood vessel destruction and serious internal organ damage. Therefore, early detection of vascular involvement could play an essential role in the diagnostic procedures of MCTD. Nailfold capillaroscopy (NFC) is widely used as a simple, non-invasive technique for investigating microvascular involvement in rheumatic diseases .
Objectives To describe the course of MCTD and its relation to nailfold capillaroscopy patterns.
Methods We analyzed clinical picture and nailfold capillaroscopy patterns (NFC) in 69 patients (pts) with MCTD recognized according to Kasukawa criteria. Clinical data achieved on the day of examination were compared with the data from beginning of the disease collected retrospectively. NFC was performed on the day of examination and analysed by one investigator. NFC changes were classified as normal (grade 0), mild (grade 1), moderate and severe (grade 3, 4). We assessed correlation of NFC changes with clinical symptoms using Spearman’s rank correlation. Study was accepted by Local Ethics Committee.
Results Median of MCTD pts age was 45 years (16 - 66), median of disease duration: 117,8 months (10-420). The beginning of disease was mainly subacute (65%), and its course was more frequently chronically progressive (63,8%). Among features with frequency diminishing from the beginning of disease to follow-up were: arthropathy, puffy hands, SLE –like skin involvement, myositis, serositis and laboratory abnormalities: haematological, hypergammaglobulinaemia and elevation of inflammatory indicators- ESR, CRP. Features with increasing prevalence in the course of the disease were: scleroderma-like skin involvement, interstitial lung disease, secondary Sjögren’s syndrome, pulmonary arterial hypertension. A Raynaud’s phenomenon was observed with similar frequency at disease onset and follow-up. Median of ANA titer was 1:5 840 (1:80 - 1:40 960). Anti-U1 RNP revealed in 98,5% of pts, anti-68kD – in 79,4%, anti-A in 88,2% and anti-C in 80,9% of pts. A NFC abnormality grade 1 was detected in 58% pts, grade 2 and 3 in 17.6%. No abnormalities were observed in 23.5% pts (grade 0). We did not find any correlation between NFC pattern and any clinical and serological features.
Conclusions Our data confirm that the beginning of MCTD is subacute with mild and moderate symptoms. Later, with disease course severe organ involvement and Sjögren’s syndrome occurred, what justifies regular screening and close monitoring of the MCTD patients. Capillaroscopy abnormalities are independent feature but, opposite to systemic sclerosis, they did not correlate with severity of organ involvement.
Furtado RN, Pucinelli MC, Cristo VV et al. Sleroderma-like nailfold capillaroscopic abnormalities are assiciated with anti-U1-RNP antibodies and Raynaud’s phenomenon in SLE patients. Lupus 2002;11:35-41.
Disclosure of Interest None Declared